Đerić Dragoslava, Dudvarski Zoran, Cvorović Ljiljana
Med Pregl. 2016 Jan-Feb;69(1-2):45-7.
Turner syndrome is a chromosomal abnormality where all or a part of one of the X chromosomes is absent or it has other abnormalities. Besides characteristic abnormalities of short stature and infertility, women with Turner syndrome have increased risks for tumors of the central nervous system, especially meningioma and an otologic disease. Meningioma involving the middle ear is extremely rare, and this condition has never been published in association with Turner syndrome.
We present an otologic manifestation associated with other abnormalities in a patient with Turner syndrome and discuss diagnosis and possible treatment options.
Multidisciplinary team approach is essential in these patients in order to evaluate their vulnerability and define therapeutic priorities.
特纳综合征是一种染色体异常疾病，其中一条X染色体全部或部分缺失，或存在其他异常。除了身材矮小和不孕等特征性异常外，特纳综合征女性患中枢神经系统肿瘤尤其是脑膜瘤和耳科疾病的风险增加。累及中耳的脑膜瘤极为罕见，且这种情况从未与特纳综合征相关联报道过。
我们报告一例特纳综合征患者伴有耳部表现及其他异常情况，并讨论其诊断及可能的治疗方案。
对于这些患者，多学科团队协作方法至关重要，以便评估其易损性并确定治疗重点。
